Bicuspid aortic valve supporting supravalvular “substrate” for multiple ventricular tachycardias Author’s Accepted Manuscript Bicuspid aortic valve supporting supravalvular “substrate” for multiple ve[.]
Author’s Accepted Manuscript Bicuspid aortic valve supporting supravalvular “substrate” for multiple ventricular tachycardias Saurabh Kumar, William G Stevenson, Usha B Tedrow www.elsevier.com/locate/buildenv PII: DOI: Reference: S2214-0271(16)30111-7 http://dx.doi.org/10.1016/j.hrcr.2016.09.006 HRCR296 To appear in: HeartRhythm Case Reports Cite this article as: Saurabh Kumar, William G Stevenson and Usha B Tedrow, Bicuspid aortic valve supporting supravalvular “substrate” for multiple ventricular tachycardias, HeartRhythm Case Reports, http://dx.doi.org/10.1016/j.hrcr.2016.09.006 This is a PDF file of an unedited manuscript that has been accepted for publication As a service to our customers we are providing this early version of the manuscript The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain Bicuspid Aortic valve Supporting Supravalvular “Substrate” for Multiple Ventricular Tachycardias Saurabh Kumar, BSc(Med)/MBBS, PhD1, William G Stevenson, MD1, Usha B Tedrow, MD1 Cardiac Arrhythmia Service, Cardiovascular Division, Brigham and Women’s Hospital, Boston, MA, USA Short title: Bicuspid aortic valve and VT Corresponding Author: Dr Usha Tedrow, MD, Cardiac Arrhythmia Service, Cardiovascular Division, Brigham and Women’s Hospital, 75 Francis St, Boston, MA- 02115 USA; Telephone: +1857-307-1947; Fax: +1857-307-1944; email: utedrow@partners.org Conflicts of Interest and Funding Sources Dr Kumar is a recipient of the Neil Hamilton Fairley Overseas Research scholarship co-funded by the National Health and Medical Research Council and the National Heart Foundation of Australia; and the Bushell Travelling Fellowship funded by the Royal Australasian College of Physicians Dr Tedrow receives consulting fees/honoraria from Boston Scientific Corp and St Jude Medical and research funding from Biosense Webster, Inc., and St Jude Medical Dr William Stevenson is coholder of a patent for needle ablation that is consigned to Brigham and Women’s Hospital Keywords: Bicuspid aortic valve, ventricular tachycardia, radiofrequency ablation, aortic commissures, left ventricular outflow tract Abbreviations ICE-intracardiac echocardiography ms-milliseconds PVC-premature ventricular contractions VT-ventricular tachycardia Introduction The great arteries are an important source of arrhythmias including premature ventricular contractions (PVC), ventricular tachycardia (VT), atrial tachycardia, and accessory pathways.1 The source of ventricular arrhythmias is thought to be muscular fibers extending from the outflow tracts to a variable extent above the valves.2 Ventricular arrhythmias from the aortic cusps exhibit complex patterns of activation breakout attributed to preferential conduction along complex insulated myocardial fibers; complexity in regional anatomy also make mapping and ablation challenging 3, We present a unique case of bicuspid aortic valve harboring the necessary “substrate” for multiple re-entrant VTs above and below the aortic valve in a patient with ischemic cardiomyopathy whose VTs where unrelated to underlying infarct substrate Case Report A 61 year old man with prior inferior wall myocardial infarction, coronary bypass grafting and ischemic cardiomyopathy (ejection fraction 40%), was referred for catheter ablation of sustained monomorphic ventricular tachycardia (VT) after experienced recurrent defibrillator shocks refractory to sotalol and amiodarone He had undergone repair of a dilated ascending aorta at the time of his bypass grafting; bicuspid aortic valve was also present but without dysfunction hence not replaced at the time of surgery Pre-procedural stress imaging showed an inferior myocardial infarct without ischemia; transthoracic echocardiography showed absence of bicuspid valvular stenosis or regurgitation and coronary angiography showed patent grafts In the electrophysiology lab, two VTs were repeatedly inducible with programmed ventricular stimulation, and with catheter manipulation within the fused cusps (Figure 1A) VT1 was right bundle morphology in lead V1, inferior axis, with a cycle length [CL] of 400 ms and was consistent with the spontaneous clinical VT VT had a qr morphology in lead V1, right inferior axis with a CL of 465 ms and was not seen spontaneously The left ventricle and aortic root were mapped retrogradely using an irrigated catheter (Thermocool SurroundFlow, Biosense Webster, Diamond Bar, CA, USA) guided by intracardiac echocardiography (ICE) integrated into the CARTO mapping system (Biosense Webster) The left and right coronary cusps were fused (Figure 1B, Movie 1) A preponderance of late potentials were noted in sinus rhythm within the fused leftright cusp, especially at the commissure where pace mapping produced near-perfect matches for both VT and VT with long stimulus-QRS delays (>80 ms) (Figure 1C, D) Coronary angiography showed absence of coronary anomalies and proposed site of ablation was >1 cm from the left main ostium (Figure 1E) During induction of VT1, a long duration, fractionated electrogram was noted within the common cusp with unipolar signals showing a QS pattern and a sharp downward deflection (Figure 1F) Radiofrequency ablation at this site terminated VT1 and rendered both VTs non-inducible (Figure 1G) Additional ablation lesions were delivered in the adjacent region within the sinuses of the fused cusps at sites that captured with pacing (10mA, 2ms) rendering the region electrically inexcitable with pacing and abolishing the late potentials within the commissure The subvalvular region was also mapped showing a small region of peri-aortic low voltage region consistent with scar, however pace maps at these sites did not resemble either VT1 or VT Some empiric ablation was performed at sites that capture with long stimulus-QRS delays However as the best pace maps and late potentials were seen in the supravalvular region, ablation was focused in this area Repeat Doppler echocardiography of the aortic valve showed no evidence of regurgitation The patient had recurrence of VT1 one month later which was successfully ablated in the periaortic left ventricular outflow tract immediately below the site of ablation in the cusps in the prior procedure At this site, attempts at entrainment of VT prior to ablation demonstrated termination without global capture (third beat as shown by arrow in Figure 1H; green dot representing this site shown in Figure 1I) The patient remained free of VT at 1-year follow up Discussion This case illustrates firstly, that the fused aortic commissures in bicuspid aortic valves are also capable providing the necessary to form the “supravalvular substrate” for multiple re-entrant sustained monomorphic VTs, akin to that reported to occur in otherwise normal trileaflet valves The mechanism is likely muscular extension above the valve cusps from the left ventricular outflow tract Second, patients with ischemic cardiomyopathy may develop ventricular arrhythmia due to substrate unrelated to the prior infarction The aortic cusps are well recognized as a source of PVC and VT.1, Sleeves of myocardium commonly extend for a variable length above the semilunar valves to the great arteries.3 Extension is more common in the right (54% in one autopsy study) than the left cusp (24%).2 Lack of inhibitive electrotonic interactions from surrounding and remote cardiomyocytes due to scarcity of muscle bundles above the semilunar valves may promote ventricular ectopy.6, Intervening regions of fibrosis, for example, in bicuspid valves may further inhibit this electrotonic interaction Prior reports of VT arising from within the cusps and immediately below the valve, suggest the presence of substrate capable of re-entry in and around the aortic commissures.4, This is the first reported case of supravalvular “substrate” supported by the presence of a bicuspid aortic valve with a fused left and right commissures, likely mediated by muscular extensions from the left ventricular outflow tract above the fused commissure We found a preponderance of late potentials within the common cusp where pacing captured with long stimulus-QRS delays providing evidence for slow conduction and produced near-identical pace map matches to the induced VT VTs were reliably inducible and terminated with programmed stimulation; and ablation terminated >1 VT at this site Moreover, low amplitude fractionated electrograms were present during induced VT with ablation at these sites terminated the VTs, rendering it noninducible.9 In addition, termination without global capture during the second procedure suggested that the region immediately below the valve in the outflow tract was also a critical area of the likely re-entry circuit, as has been previously shown in patients with prior coronary disease.10 Although limited empiric ablation was performed in the subvalvular region in the first procedure, it is unclear if extensive ablation through the low voltage peri-aortic region would have avoided a subsequent procedure by eliminating all potential exits of the VTs It is plausible that generous supravalulvar myocardial extension from fusion of the right and left cusps where myocardial extension is the most prominent, when combined with the enhanced fibrosis commonly seen in fused cusps promoted the development of substrate for re-entry in this patient Pacing at one site in the fused commissures, replicated two different pace map morphologies of VT This is consistent with the finding that multiple exits of VT are possible attributed to preferential conduction along myocardial fibers travelling to surrounding structures such as the right ventricular outflow tract, epicardial left ventricular summit, great cardiac vein, and the infravalvular left ventricular outflow tract, which often makes pace-mapping unreliable in the supravalvular left ventricular outflow tract.11 Lastly, the presence of outflow tract arrhythmia in this patient also illustrates that not all ventricular arrhythmias may arise from anticipated regions of substrate providing by prior infarction.12 In one study, patients with coronary artery disease and VT, regions of abnormal voltage giving rise to VT had a basal perivalvular distribution or epicardially remote from anticipated infarction sites, suggesting the presence of concurrent non-ischemic substrate for VT In conclusion, this case provides electrophysiologic data and anatomical imaging to illustrate the presence of substrate above and below the valve for multiple VTs in a unique patient with a fused bicuspid valve, and second, it highlights the importance of recognizing that ventricular arrhythmias may arise from regions unrelated to anticipated substrate of prior infarction in patients with ischemic cardiomyopathy References Tabatabaei N, Asirvatham SJ Supravalvular arrhythmia: identifying and ablating the substrate Circ Arrhythm Electrophysiol 2009;2:316-326 Gami AS, Noheria A, Lachman N, Edwards WD, Friedman PA, Talreja D, Hammill SC, Munger TM, Packer DL, Asirvatham SJ Anatomical correlates relevant to ablation above the semilunar valves for the cardiac electrophysiologist: a study of 603 hearts J Interv Card Electrophysiol 2011;30:5-15 Yamada T, McElderry HT, Doppalapudi H, et al Idiopathic ventricular arrhythmias originating from the aortic root prevalence, electrocardiographic and electrophysiologic characteristics, and results of radiofrequency catheter ablation J Am Coll Cardiol 2008;52:139-147 Yamada T, Platonov M, McElderry HT, Kay GN Left ventricular outflow tract tachycardia with preferential conduction and multiple exits Circ Arrhythm Electrophysiol 2008;1:140142 Yamada T, McElderry HT, Doppalapudi H, Okada T, Murakami Y, Yoshida Y, Yoshida N, Inden Y, Murohara T, Plumb VJ, Kay GN Idiopathic ventricular arrhythmias originating from the left ventricular summit: anatomic concepts relevant to ablation Circ Arrhythm Electrophysiol 2010;3:616-623 Takayanagi K, Nakahara S, Toratani N, Chida R, Kobayashi S, Sakai Y, Takeuchi A, Ikeda N Strong modulation of ectopic focus as a mechanism of repetitive interpolated ventricular bigeminy with heart rate doubling Heart Rhythm 2013;10:1433-1440 Bradfield JS, Homsi M, Shivkumar K, Miller JM Coupling interval variability differentiates ventricular ectopic complexes arising in the aortic sinus of valsalva and great cardiac vein from other sources: mechanistic and arrhythmic risk implications J Am Coll Cardiol 2014;63:2151-2158 Yamada T, McElderry HT, Doppalapudi H, Kay GN Ventricular tachycardia with a myocardial fibre travelling from the origin in the right aortic sinus cusp to the epicardial breakout site of the right ventricular outflow tract Europace 2008;10:469-470 Stevenson WG, Friedman PL, Sager PT, Saxon LA, Kocovic D, Harada T, Wiener I, Khan H Exploring postinfarction reentrant ventricular tachycardia with entrainment mapping J Am Coll Cardiol 1997;29:1180-1189 10 Bogun F, Hohnloser SH, Bender B, Li YG, Groenefeld G, Pelosi F, Oral H, Knight B, Strickberger SA, Morady F Mechanism of ventricular tachycardia termination by pacing at left ventricular sites in patients with coronary artery disease J Interv Card Electrophysiol 2002;6:35-41 11 Yamada T, Murakami Y, Yoshida N, et al Preferential conduction across the ventricular outflow septum in ventricular arrhythmias originating from the aortic sinus cusp J Am Coll Cardiol 2007;50:884-891 12 Aldhoon B, Tzou WS, Riley MP, Lin D, Callans DJ, Hutchinson MD, Dixit S, Garcia FC, Zado ES, Marchlinski FE Nonischemic cardiomyopathy substrate and ventricular tachycardia in the setting of coronary artery disease Heart Rhythm 2013;10:1622-1627 Figure Legend (A) Induced VT1 and V2; (B) fused left and right commissures on intracardiac echocardiography; (C) Excellent pace match matches for both VTs with long stimulus-QRS delay at sites with late potentials (D) in the left-right fused bicuspid commissure > cm below the left main coronary ostium (E) where long duration fractionated signal with a unipolar QS was seen during VT (F, G) VT recurred month later and entrainment attempt at the periaortic left ventricular outflow tract below the site of previous ablation showed termination without global capture (arrow, H; site in green in I) Despite a prior inferior wall infarction, there was peri-aortic low voltage with minimal inferior wall scar, more consistent with a non-ischemic distribution of scar 10 .. .Bicuspid Aortic valve Supporting Supravalvular “Substrate” for Multiple Ventricular Tachycardias Saurabh Kumar, BSc(Med)/MBBS, PhD1, William... challenging 3, We present a unique case of bicuspid aortic valve harboring the necessary “substrate” for multiple re-entrant VTs above and below the aortic valve in a patient with ischemic cardiomyopathy... illustrates firstly, that the fused aortic commissures in bicuspid aortic valves are also capable providing the necessary to form the ? ?supravalvular substrate” for multiple re-entrant sustained monomorphic